Flashcards in Lung Deck (452)
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Congenital pulmonary-airway malformation: Definition.
Malformation of part of the lung parenchyma, with abnormal direct connections to the normal tracheobronchial tree.
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Congenital pulmonary-airway malformation: Blood supply.
Derived from the pulmonary circulation.
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Congenital pulmonary-airway malformation, type 0: Gross pathology.
Lung parenchyma appears solid.
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Congenital pulmonary-airway malformation, type 0: Histology (2).
Disorganized proximal airways; no distal airways.
Intervening tissue consists of connective tissue, thick-walled arteries, and extramedullary hematopoiesis.
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Congenital pulmonary-airway malformation, type 1: Gross pathology.
Medium and large interconnecting cysts, usually limited to one lobe.
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Congenital pulmonary-airway malformation, type 1: Histology.
Cysts are lined by bronchial epithelium and often contain bands of smooth muscle.
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Congenital pulmonary-airway malformation, type 2: Gross pathology.
Back-to-back cysts, smaller than the cysts of type 1.
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Congenital pulmonary-airway malformation, type 2: Histology.
Cysts are separated by alveolar ductlike structures, blood vessels, skeletal muscle.
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Congenital pulmonary-airway malformation, type 2: Associations.
Sirenomelia.
Renal malformations.
Diaphragmatic hernia.
Cardiovascular abnormalities.
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Congenital pulmonary-airway malformation, type 3: Gross pathology.
Solid mass involving the lobe or the entire lung, with hypoplasia of the other lung.
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Congenital pulmonary-airway malformation, type 3: Histology.
Glandlike structures lined by low cuboidal epithelium.
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Congenital pulmonary-airway malformation, type 3: Associations.
Male sex.
Polyhydramnios.
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Congenital pulmonary-airway malformation, type 4: Gross pathology.
Peripherally located cysts of variable size.
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Congenital pulmonary-airway malformation, type 4: Histology.
Cysts can have thick walls and are lined by a single layer of pneumocytes.
Capillary beds beneath the epithelial lining.
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Congenital pulmonary-airway malformation, type 4: IHC.
Pneumocytes express TTF-1 and surfactant proteins A and B.
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Congenital pulmonary-airway malformation: Limitations on diagnosis.
Diagnosis cannot be made when there is chronic inflammation and fibrosis.
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Bronchopulmonary sequestration: Definition (2).
Anomalous segment of lung has no connection with the normal tracheobronchial tree.
Blood supply comes from the systemic circulation.
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Bronchopulmonary sequestration: Types (2).
Intralobar.
Extralobar.
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Bronchopulmonary sequestration, intralobar type: Typical location.
Lower lobe.
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Bronchopulmonary sequestration, intralobar type: Gross pathology (3).
No pleural covering.
Sharply demarcated from the normal lung tissue.
May have vascular pedicle.
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Bronchopulmonary sequestration, intralobar type: Histology (3).
Marked chronic inflammation.
Mucus accumulation and microcyst formation.
Dense fibrosis surrounds remnants of bronchioles.
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Bronchopulmonary sequestration, extralobar type: Typical location.
Left side.
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Bronchopulmonary sequestration, extralobar type: Gross pathology (2).
Covered with visceral pleura.
Separate from normal lung tissue.
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Bronchopulmonary sequestration, extralobar type: Histology (3).
Enlarged bronchi, bronchioles, alveoli.
No significant inflammation or fibrosis.
Dilated subpleural lymphatics.
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Bronchopulmonary sequestration, extralobar type: Association.
Congenital pulmonary-airway malformation, type 2, is present in up to half of cases.
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Bronchogenic cyst: Origin.
Anomalous budding of the tracheobronchial anlage during development.
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Bronchogenic cyst: Most common site.
Mediastinum.
No communication with the tracheobronchial tree.
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Bronchogenic cyst: Radiography.
May have an air-fluid level.
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Bronchogenic cyst: Histology (3).
Epithelial lining and wall (smooth muscle, cartilage) resemble those of a normal bronchus.
Squamous metaplasia or chronic inflammation may occur.
No alveolar tissue.
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